Endoscopic evaluation and management of hereditary pancreatitis

Abstract

Ever since an association between Trypsinogen mutations and the phenotype of hereditary pancreatitis have been discovered in 1996, this disease variety has been recognized as a pathophysiological challenge and a vexing clinical problem. Affected patients differ from patients with other types of chronic pancreatitis in that they rarely drink alcohol, that the disease process begins early in life and most often affects children and young adults at the time of initial diagnosis, and that there is no removable causative factor and therefore the disease invariably progresses. In this light, we review the available data on endoscopic evaluation and management of affected patients with a particular focus on the early detection of pancreatic cancer, for which the risk in hereditary pancreatitis patients is manifold increased, and on the options for endoscopic treatment of the characteristic complications of the disorder.